Sickle cell anaemia 2: management approaches of painful episodes.
ABSTRACT The first of this two-part series highlighted how sickle cell disorders were the most common genetic disorder in the UK. It also described how recurrent sickle cell painful episodes led to many social restrictions on a sufferer's life. In the UK, painful episodes still remain the main reason for the hospital admission of people with sickle cell disease. This often means lengthy periods spent as an inpatient for many sufferers, as well as a lifetime reliant upon health professionals to prescribe strong analgesics in an attempt to try and control the painful symptoms associated with sickle cell disease. Readers should note that the term 'painful episode' is now used in preference to 'crisis'. Despite recommendations that initial hospital management of sickle cell painful episode should be aimed at providing rapid pain control, this article will show that choosing the right analgesic is not always easy to decide. National guidelines may aid this process, incorporated alongside increased patient empowerment and the important role of the nurse.
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ABSTRACT: This critical literature review aims at demonstrating the state of the art of a thematic family with a sickle cell disease child and the nursing team. The PubMed, Lilacs, SciELO, and BDENF databases were searched using the key words: sickle cell anaemia, nursing, family and child. Eleven articles were selected and the important points in respect to the quanti-qualitative aspects of the studies are described. A referential analysis of the content was performed which identified the following themes: scientific knowledge, nursing care and education. The necessity of specific knowledge about sickle cell disease was demonstrated: this is important for nursing care to be effective and contribute to a better quality of life and so that the survival of these children is prolonged. It is worth noting that the Brazilian production on sickle cell disease, in general, is scarce and incipient in particular in respect to studies about the family with a sickle cell disease child and nursing teamRevista Brasileira de Hematologia e Hemoterapia 12/2009; 32(3):257-264.
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ABSTRACT: Patients undergoing allogeneic stem cell transplants (SCT) are at increased risk for vitamin D deficiency through prolonged hospitalizations and avoidance of sunlight as well as decreased nutritional status and malabsorption related to an allogeneic SCT. Vitamin D deficiency was shown to be as high as 39% in the pretransplant setting  and 90% in the postallogeneic transplant setting . The purpose of this study was to confirm the high incidence of vitamin D deficiency in the pre and postallogeneic transplant setting, the demographic factors associated with vitamin D deficiency, and the correlation between vitamin D deficiency and osteopenia and osteoporosis in patients undergoing allogeneic stem cell transplantation. We found that 70% of patients were vitamin D deficient at day 0 of an allogeneic SCT, and 58% of patients were vitamin D deficient at post-transplant day 100. The incidence of osteopenia and osteoporosis in day 100 of the post-transplant setting was 83% and 22%, respectively, however there was no difference in incidence of osteopenia or osteoporosis in patients who were vitamin D deficient or nondeficient. Our study confirms the high incidence of vitamin D deficiency in patients undergoing allogeneic transplant patients.American Journal of Hematology 11/2011; 86(11):954-6. DOI:10.1002/ajh.22143 · 3.48 Impact Factor
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ABSTRACT: Despite the benefits of hydroxycarbamide (HU) for sickle cell disease (SCD) patients, it is currently underutilized in routine clinical management because of concerns about effectiveness and safety and ambivalence surrounding optimal HU regimes for heterogeneous SCD clinic populations [1-6]. This UK study followed up 62 SCD patients treated with HU for up to 9 years (IQR 1-6 yrs). Patients benefited from sustained significant increases in mean annual hemoglobin (Hb), fetal hemoglobin (HbF%), mean cell volume (MCV) and reduction in absolute neutrophil count (ANC), mean annual inpatient (IP) days, acute chest syndrome (ACS) and transfusion events compared to pretreatment. Mean daily dose and maximum tolerated dose (MTD) achieved were constrained by patients' concordance with treatment and drug tolerance. All serious adverse events were not prevented during therapy. Side effects did not warrant cessation of HU but deteriorating medical condition did. Long-term treatment warrants close monitoring with multiagency involvement in care.American Journal of Hematology 11/2011; 86(11):958-61. DOI:10.1002/ajh.22146 · 3.48 Impact Factor